Onco-neural antibodies and tumour type determine survival and neurological symptoms in paraneoplastic neurological syndromes with Hu or CV2/CRMP5 antibodies.
OBJECTIVE
Anti-Hu antibodies (Hu-Ab) and anti-CV2/CRMP5 antibodies (CV2/CRMP5-Ab) have been identified in association with paraneoplastic neurological disorders. However, it is not clear whether these antibodies are associated with specific neurological symptoms or are only markers of anti-cancer immune reaction.


METHODS
To address this question, 37 patients with CV2/CRMP5-Ab and 324 patients with Hu-Ab were compared.


RESULTS
Whereas the age and sex ratio were the same between the two groups, the distribution of neurological symptoms was not. Patients with CV2/CRMP5-Ab presented more frequently cerebellar ataxia, chorea, uveo/retinal symptoms and myasthenic syndrome (Lambert-Eaton myasthenic syndrome LEMS or myasthenia gravis). They also had a better Rankin score. In contrast, dysautonomia, brainstem encephalitis and peripheral neuropathy were more frequent in patients with Hu-Ab. Limbic encephalitis occurred similarly in both groups. Small-cell lung cancer was the most frequently associated tumour in both groups of patients, while malignant thymoma was observed only in patients with CV2/CRMP5-Ab. In particular, patients with CV2/CRMP5-Ab and thymoma developed myasthenic syndrome more frequently, while patients with SCLC developed neuropathies more frequently. Chorea and myasthenic syndrome were only seen in patients with CV2/CRMP5-Ab. The median survival time was significantly longer in patients with CV2/CRMP5-Ab, and this effect was not dependent on the type of tumour.


CONCLUSIONS
The data demonstrate that in patients with paraneoplastic neurological syndromes, the neurological symptoms and survival vary with both the type of associated onco-neural antibody and the type of tumour.
Honnorat
J
J
Centre de Référence Maladies Rares Syndromes Neurologiques Paranéoplasiques, Hospices Civils de Lyon, Hôpital Neurologique, Bron, France. jerome.honnorat@chu-lyon.fr
Cartalat-Carel
S
S
Ricard
D
D
Camdessanche
J Ph
JP
Carpentier
A F
AF
Rogemond
V
V
Chapuis
F
F
Aguera
M
M
Decullier
E
E
Duchemin
A M
AM
Graus
F
F
Antoine
J C
JC
D000328:Adult; D017668:Age of Onset; D000368:Aged; D000912:Antibodies, Neoplasm / Q000276:immunology; D001932:Brain Neoplasms / Q000453:epidemiology / Q000276:immunology* / Q000473:pathology*; D051959:ELAV Proteins / Q000276:immunology*; D005260:Female; D006801:Humans; D006867:Hydrolases; D008297:Male; D008869:Microtubule-Associated Proteins; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology*; D009422:Nervous System Diseases / Q000209:etiology / Q000503:physiopathology; D020361:Paraneoplastic Syndromes, Nervous System / Q000453:epidemiology / Q000276:immunology* / Q000473:pathology*; D011379:Prognosis; D016019:Survival Analysis; D013945:Thymoma / Q000473:pathology
D000912:Antibodies, Neoplasm; D051959:ELAV Proteins; D008869:Microtubule-Associated Proteins; D009419:Nerve Tissue Proteins; C415087:DPYSL5 protein, human; D006867:Hydrolases